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What is Stiff Person Syndrome SPS a rare neurological disorder? What are it’s cause ? celine Dion affected by it

Dec 9, 2022

Celine Dion has revealed that she was diagnosed with Stiff Person Syndrome (SPS), a rare and progressive neurological disorder. The 54-year-old singer shared an emotional Instagram video about living with a condition that makes her muscles spasm uncontrollably and affects “every aspect” of her day-to-day life.

“I’ve been dealing with problems with my health for a long time,” said Dion.

“Recently I’ve been diagnosed with a very rare neurological disorder called the stiff person syndrome which affects something like one in a million people.

“While we’re still learning about this rare condition, we now know this is what’s been causing all of the spasms I’ve been having,” she said.Dion added that she would have to reschedule her Europe tour, set to begin in February, so she could better focus on her health. “It hurts me to tell you today that this means I won’t be ready to restart my tour in Europe in February,” the Titanic singer said.

What is Stiff Person Syndrome?

Stiff Person Syndrome (SPS), also called Stiff Man Syndrome (SMS), is a rare neurological disorder which causes progressive stiffness, rigidity and muscle spasms.

People affected by the disorder experience rigidity in their limbs and torso. The stiffness can impair mobility and affect posture. Chronic pain is also a symptom of this health condition.

Aside from the stiffness, muscle spasms can sometimes be so severe they cause the affected person to fall down. These spasms can occur at random or they can be triggered by things like loud sounds or emotional distress, according to NBC. People with severe SPS are often wary of leaving their house because loud noises like horns can trigger spasms and cause them to fall.

The condition was first identified in 1956, and was dubbed stiff-man syndrome. The name has since been changed — women in fact make up a majority of cases.

Causes and management

“Scientists don’t yet understand what causes SPS, but research indicates that it is the result of an autoimmune response gone awry in the brain and spinal cord,” according to the National Institute of Neurological Disorders and Stroke.

Around one in a million people are estimated to have the condition, according to the US National Organization for Rare Disorders (NORD). Twice as many women are affected by SPS as men.

Symptoms develop over a matter of months or years, normally between the ages of 30 to 60, and can remain stable in some cases or get gradually worse in others.

There is no cure for SPS, but its symptoms can be managed. Dr. Richard Nowak, an assistant neurology professor at the Yale School of Medicine, said the condition “has a range of severity, from quite mild — easily managed with a little bit of medication — to folks that are quite severe that can be, frankly, quite disabled from it.”

Doctors may prescribe medication like muscle relaxants to people affected by Stiff Person Syndrome, which can help slow down the progression of the disorder.

(With inputs from AFP)

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